Fatal peri-operative acute tumour lysis syndrome precipitated by dexamethasone

A 3-year-old patient presented for elective adenotonsillectomy to treat symptomatic obstructive sleep apnoea. The patient had not been assessed at a pre-operative anaesthesia clinic but had undergone uneventful general anaesthesia twice in the previous two years. An uneventful operative course was complicated by the development of clinical instability over the first 6 h postoperatively culminating in cardiorespiratory arrest. Subsequent investigation demonstrated the acute development of tumour lysis syndrome in the setting of a new onset, undiagnosed acute leukaemia. The patient died on the third postoperative day. The use of dexamethasone for prophylaxis against postoperative nausea and vomiting was the likely aetiology of the acute tumour lysis syndrome in this case. This is the first documented peri-operative death due to tumour lysis syndrome after adminstration of dexamethasone. We discuss the various problems encountered with this case and review the recent literature and case reports on tumour lysis syndrome in the operating theatre.     

Tumour lysis syndrome: an unusual presentation

We report a case of spontaneous tumour lysis syndrome that developed postoperatively in a patient with undiagnosed Burkitt’s lymphoma. The former diagnosis was made, unusually, following the development of white emulsion‐like urine in the catheter bag whilst the patient was being managed in the intensive care unit. After laboratory analysis, the urine was found to contain large quantities of uric acid crystals which were the key to the prompt diagnosis. Spontaneous tumour lysis syndrome is rare and this case highlights the difficulties in making an early diagnosis when the presence of a predisposing tumour has not yet been identified. Untreated tumour lysis syndrome can be fatal due to severe biochemical disturbances causing cardiac dysfunction and multi‐organ failure. Early recognition and treatment are crucial to prevent morbidity and mortality. The unusual presentation of this case in association with an undiagnosed Burkitt’s lymphoma emphasises how vigilant anaesthetists and intensivists must be in recognising this potentially life‐threatening condition. We believe that the triggering factor in this case was laparotomy and handling of the tumour.     

Tumour lysis syndrome developing during an operation

We describe an unusual case of tumour lysis syndrome in a child with a high-grade lymphoma undergoing a staging laparotomy. The patient presented with a refractory ventricular arrhythmia, which required continuous resuscitation in the operating room and continuous venous-venous haemodialysis in the intensive care unit. This case report suggests that surgery is a possible trigger for developing tumour lysis syndrome, so anaesthetists should be alert to this possibility during surgery in patients with pre-existing high tumour burdens.     

Extracorporeal life support in the acute management of tumour lysis syndrome

A 16-month old boy presented with a severe tumour lysis syndrome (TLS) complicating induction therapy for acute myeloid leukaemia. This was further complicated by a respiratory syncytial virus infection. The failure of response to escalating treatment necessitated the use of extracorporeal life support (ECLS) during continuation of his induction chemotherapy. He was weaned from support after the resolution of the TLS and completed chemotherapy successfully. ECLS may have a role to play in the acute support of children with cardiorespiratory failure as a result of malignancy or the required treatment.     

Effects of continuous arteriovenous hemofiltration on cardiopulmonary abnormalities during anesthesia for orthotopic liver transplantation

Orthotopic liver transplantation (OLT) often involves large blood loss and replacement, as well as administration of large amounts of blood products to correct coagulation defects. Renal free water excretion is often impaired in end-stage liver disease and not responsive to routine diuretic therapy, predisposing these patients to accumulation of extravascular lung water. The effects of the intraoperative use of continuous arteriovenous hemofiltration (CAVH) on cardiopulmonary and oxygen transport variables were studied in ten patients during and 24 hours after OLT. CAVH prevented increases in pulmonary shunt fraction while decreasing PEEP and FIO2 requirements. Pulmonary compliance was significantly higher after operation in patients receiving CAVH. Before surgical dissection, hemodynamic effects of CAVH were minimal. Postoperatively, patients having CAVH had lower cardiac filling pressures and more normal systemic vascular resistance than did patients not having CAVH. These data suggest that CAVH may be a useful intraoperative technique for patients with impaired renal function undergoing liver transplantation.     

Acute renal failure from xanthine nephropathy during management of acute leukemia

Tumor lysis syndrome is a potentially life-threatening complication of induction chemotherapy for treatment of lymphoproliferative malignancies. Serious complications of tumor lysis syndrome are rare with the preemptive use of allopurinol, rasburicase, and urine alkalinization. We report a case of oliguric acute renal failure due to bilateral xanthine nephropathy in an 11-year-old girl as a complication of tumor lysis syndrome during the treatment of T-cell acute lymphoblastic leukemia. Xanthine nephrolithiasis results from the inhibition of uric acid synthesis via allopurinol which increases plasma and urinary xanthine and hypoxanthine levels. Reports of xanthine nephrolithiasis as a cause of tumor lysis syndrome are rare in the absence of defects in the hypoxanthine-guanine phosphoribosyl transferase (HGPRT) enzyme. Xanthine nephropathy should be considered in patients who develop acute renal failure following aggressive chemotherapy with appropriate tumor lysis syndrome prophylaxis. Urine measurements for xanthine could aid in the diagnosis of patients with nephrolithiasis complicating tumor lysis syndrome. Allopurinal dosage should be reduced or discontinued if xanthine nephropathy is suspected.     

Continuous arteriovenous hemofiltration in the treatment of 100 critically ill patients with acute renal failure: report on clinical outcome and nutritional aspects

The clinical outcome for 100 consecutive patients with multiorgan failure including acute renal failure (ARF) was studied. Fifty-eight of the patients had acute renal failure due to complications during and after major surgery. Seventy-three of the patients had a urine output of less than 400 ml/24 hours. The majority of the patients also had complications such as septicemia or respiratory insufficiency and required vasopressor infusions. All patients were treated with continuous arteriovenous hemofiltration (CAVH). The duration of the CAVH treatment varied between a few hours and 90 days, with a mean of 8 days. The mean ultrafiltration volume per 24 hours was, on the average, 12 liters. CAVH resulted in adequate uremic control in 89 cases, but additional treatment with intermittent hemofiltration was necessary in 11 patients. The total survival rate was 45% including survival rates as high as 54% in patients with ARF complicating abdominal aortic surgery. Only three patients were referred for chronic dialysis therapy. In a subgroup of 17 patients with ARF complicating abdominal aortic surgery the nutritional aspects during CAVH were studied. It is concluded that during CAVH therapy it is possible to give adequate nutritional support even to hypercatabolic and anuric patients.     

Treatment of hydrops fetalis with hemofiltration

The treatment of hydrops fetalis, a critical state of extravascular fluid overload in the newborn, poses a great medical challenge. The aim of this study was to investigate the use of continuous arteriovenous hemofiltration (CAVH) in the treatment of five critically ill newborns with hydrops fetalis of different etiology. All patients had anasarca, oliguria not responsive to diuretics, were on mechanical ventilation, and were treated with inotropic drugs and sedation. The duration of treatment ranged from 42 to 114 h and all patients, except one who died from congenital heart disease, achieved the clinical goals of weight loss and restoration of diuresis. Regular clinical and neurological follow-up to the age of 2 years was normal for the four surviving patients. In conclusion, CAVH appears to be a safe treatment of fluid overload in newborns with hydrops fetalis. Received: 17 September 1998 / Revised: 24 June 1999 / Accepted: 25 June 1999     

Heteroconjugated antibodies enhance lymphocyte-mediated tumour cell lysis in vitro and in vivo.

Covalent linkage of an antitumour antibody specific for a tumour cell surface antigen to an antilymphocyte antibody specific for the T lymphocyte receptor complex produces a heteroconjugated antibody that can activate and redirect cytotoxic T lymphocytes to lyse tumour cells. The ability of an antilymphocyte-antitumour heteroconjugate (500A2 x 96.5) to direct the lysis of murine melanoma cells by cultured murine lymphocytes was tested in vitro using a 4-h chromium release assay and in vivo with a tumour neutralization assay. In vitro, the addition of heteroconjugated antibody significantly increased tumour lysis by murine C3H/HeN lymphocytes (median specific lysis 82.7 per cent with lymphocytes plus heteroconjugate versus 9.5 per cent for lymphocytes alone, P less than 0.001). In vivo, treatment with heteroconjugated antibody plus lymphocytes significantly reduced the development of pulmonary metastases after intravenous tumour administration (median number of pulmonary metastases 28.5 for combined treatment versus 250 for heteroconjugate or lymphocytes alone, P less than 0.001).     

Tumour lysis syndrome during anaesthesia

We describe a child who developed acidosis, hyperkalaemia and cyanosis while emerging from an otherwise uneventful anaesthetic. A diagnosis of tumour lysis syndrome was suspected and confirmed biochemically. Although this syndrome is usually associated with chemotherapy and has been described to occur spontaneously, it has not previously been reported as being associated with anaesthesia.     

Continuous arteriovenous hemofiltration in children

Continuous arteriovenous hemofiltration (CAVH) is an extracorporeal technique for the treatment of hypervolemia and electrolyte disturbances in the critically ill patient with oligoanuria. The patient's cardiac output provides the blood flow through the circuit; no pumps are necessary. A range of hemofilters is now available extending the applicability of CAVH to the pediatric population, including premature newborns. In this report the treatment of 15 neonates and 8 older children is described. Fluid overload was reduced in all cases. Reflecting the very grave clinical conditions of these patients, 15 of the 23 treated children ultimately died. Due to failure to control uremia, four patients required treatment with dialysis. CAVH was found to be generally safe and effective even in the hemodynamically unstable critically ill child.Presented at the annual meeting of the American Society of Pediatric Nephrology, 5 May 1986, organized by Dr. R. N. Fine, Los Angeles.     

Introduction of percutaneous arteriovenous femoral shunt: a new access for continuous arteriovenous hemofiltration

In most critically ill unstable patients, continuous arteriovenous hemofiltration (CAVH) and continuous arteriovenous hemofiltration with dialysis (CAVHD) offer significant advantages over dialysis and ultrafiltration. Improvements in CAVH methodology have occurred since its introduction, but an ideal vascular access has yet to be designed. Scribner shunts may result in loss of a future access site, while femoral artery and vein catheterization render the patient immobilized. We have developed a percutaneous external arteriovenous femoral shunt to overcome this difficulty. The catheter consists of two single-lumen flexible polyurethane catheters with a subcutaneous Dacron cuff. A percutaneous Seldinger technique is used with a peel-away sheath to insert one catheter in the artery and one in the vein. To minimize infection and ensure durability, a 10-cm tunnel is created so that the exit of the catheters on the upper thigh is away from the groin. Like the Scribner shunt, a connecting tube is used between the catheters to maintain the patency when the shunt is not in use. Our experience with this technique is limited to seven treatments in seven patients (five CAVH, two CAVHD). Good blood flow (average, 80 mL/min), ultrafiltration rate (7 mL/min), and biochemical studies demonstrated the efficiency of the access. The average treatment was 10 days and patients were allowed to do moderate movement without resulting thrombosis or infection of the access. The major advantages of this new and efficient access for CAVH and CAVHD include simple introduction by a nephrologist, lack of potential serious complications, avoidance of sacrifice of major vessels, early ambulation and movement without compromising function, and a design for prolonged usage.     

Continuous arteriovenous hemofiltration in the newborn

Acute renal failure (ARF) in newborns and infants is a serious postoperative complication. Peritoneal dialysis is considered the treatment of choice, but is often impossible postoperatively. In adult patients or older children, continuous arteriovenous hemofiltration (CAVH) has been established for 10 years. In 1985, Ronco published case reports on four newborn infants treated with CAVH using a specially designed hemofilter. In between, a few more case reports have been published. We report a case of postoperative septic ARF in an 8-week-old girl with a body weight of 4000 g. We used the Minifilter (Amicon Corp.) connected to a femoral arterial Shaldon catheter and initially a central venous line, later a subclavian Shaldon catheter. Intermittent hemodialysis without ultrafiltration was performed as well. With ultrafiltration rates of 2 to 24 ml/h, mean 12.8 ml/h, we achieved an impressive loss of weight and the pulmonary function improved. The baby died on the 5th day of CAVH treatment of septic multiorgan failure. Even though the small patient did not survive, we demonstrated the feasibility of the method in newborns. CAVH is recommended for overhydrated babies with oligoanuric ARF, but it is not sufficient as a substitute for dialysis in these patients.     

Anaesthetic management of hepatic artery chemoembolization in a paediatric patient

We present a case of a 3-year-old child who underwent hepatic artery chemoembolization. The anaesthetic management, prophylaxis of tumour lysis syndrome, nausea and vomiting and the management of perioperative pain relief are all discussed.     

Partial hypoxanthine-guanine phosphoribosyltransferase deficiency presenting as acute renal failure

Hyperuricemia and secondary urate nephropathy are uncommon in the paediatric setting outside of tumour lysis syndrome. We describe the case of a 12-year-old boy who presented at 3 years of age with acute renal failure. The cause of this remained unknown until the development of uric acid renal calculi 9 years later. This, and the availability of the previously unknown family history, provided the subsequent diagnosis of partial hypoxanthine-guanine phosphoribosyltransferase (HPRT) deficiency. Detailed family history is important for early detection of this heterogeneous group of disorders. Early treatment may minimise long-term renal morbidity and mortality from renal insufficiency.     

Continuous arteriovenous hemofiltration in acute renal failure

This extensive review describes the settings for continuous arteriovenous hemofiltration (CAVH) and attempts to compare it to traditional dialysis therapies for acute renal failure. In addition hemodynamic stability, membrane biocompatibility, nutrition, fluid and solute removal, operational characteristics, anticoagulation, replacement solutions, drug removal, complications, and trouble shooting during CAVH are all discussed in detail. The cost of CAVH v dialysis is equal. CAVH is probably the renal replacement therapy of choice for hemodynamically unstable patients with acute renal failure and contraindications to peritoneal dialysis.     

Continuous arteriovenous haemofiltration in the newlyborn with acute renal failure and congenital heart disease

Newlyborn infants with congenital heart disease who develop acute renal failure are particularly difficult to treat. There are often complex associated medical problems and the mortality is high. Continuous arteriovenous haemofiltration (CAVH) provides a slow and gentle removal of fluid, together with the possibility of correcting metabolic abnormalities. We used CAVH in six newlyborn infants all with severe congenital heart disease, who developed acute renal failure early in life. In four patients it was necessary to insert a blood pump into the circuit to maintain adequate blood flow. CAVH alone, with or without a blood pump, was unable to reduce the plasma urea and creatinine, and in three of the infants, dialysis across the filter was required. CAVH was effective in controlling fluid balance. Although mortality remains high we feel CAVH has an important role in selected patients.     

Continuous renal replacement therapy: opinions and evidence

Continuous arteriovenous haemofiltration (CAVH) is the first example of continuous renal replacement therapy (CRRT). CAVH was first applied for the treatment of diuretic unresponsive fluid overload. Subsequently, CRRT has undergone a remarkable growth, and it is now performed with pump technology (CVVH) and via double-lumen central venous catheters. In many intensive care units, especially in Australia and in Europe, CRRT has become the dominant, if not exclusive, form of artificial renal support. Continuous haemofiltration is now used beyond the original indications of blood purification, for the treatment of certain drug intoxications, for severe cardiac failure, for volume control during, after cardiopulmonary bypass, and to decrease the toxicity of chemotherapy. Furthermore, there is strong ongoing research into its role or that of derived techniques as possible adjuvant therapies during severe sepsis. Despite its large use, the current state of CRRT is surrounded by some controversies, and an effort should be made to give a dispassionate distillation of the literature for a final common definition of what is based on opinions and what carries sufficient evidence.     

Continuous arteriovenous hemodiafiltration in the acute treatment of hyperammonaemia due to ornithine transcarbamylase deficiency

BACKGROUND: Acute hyperammonemia caused by urea cycle disorder is a medical emergency for which immediate managements should be taken to minimize permanent brain damage. Among different enzyme defects, ornithine transcarbamylase deficiency (OTC) is one of the most common enzyme defect in urea cycle disorders. We utilized continuous renal replacement therapy techniques in the acute treatment of hyperammonemia due to ornithine transcarbamylase deficiency. PATIENTS AND METHODS: Three male neonates with elevated serum ammonia levels were shown, based on urine organic acid analysis and serum amino acid studies, to have OTC deficiency. Administration of sodium benzoate and sodium phenylacetate for activating alternative nitrogen waste pathway were used associated with protein restriction. Other modalities, including blood exchange transfusion, peritoneal dialysis, continuous renal replacement therapy were utilized in an attempt to lower serum ammonia concentration. RESULTS: We report the successful use of continuous arteriovenous hemofiltration (CAVH), continuous arteriovenous hemodialysis (CAVHD), continuous arteriovenous hemodiafiltration (CAVHDF) in the acute management of hyperammonemia due to OTC deficiency. We also compared the ammonia clearance between peritoneal dialysis, exchange transfusion, CAVH, CAVHD and CAVHDF. It demonstrated the evidence that CAVHDF provides the best ammonia clearance. CONCLUSION: Continuous renal replacement therapy including CAVH, CAVHD, and CAVHDF may be the alternative techniques for acute management of hyperammonemia in inborn error of metabolism when dialysis machine is not available. Our data suggests CAVHDF provides the best ammonia clearance.     

Intra-abdominal metastasis of an intracranial germinoma via ventriculo-peritoneal shunt in a 13-year-old female

A 13-year-old patient presented with massive intra-abdominal metastasis and spontaneous acute tumour lysis syndrome, 17-months after VP shunt placement for metastatic pineal germinoma treated with cranio-spinal-irradiation. Hyperhydration/rasburicase improved renal function, allowing chemotherapy with subsequent surgery. The patient remains event-free 34-months later. Risk of intra-abdominal metastasis from VP shunts is discussed.